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488 Chapter 12: Haematology and clinical immunology

Table 12.7 Staging of CLL Lymphoma
Stage 0 Lymphocytosis of blood and marrow
Stage I Lymphocytosis and lymphadenopathy Hodgkin’s disease
Stage II Lymphocytosis with enlarged liver or spleen
Stag III Stage 0–II with haemoglobin <11 g/dL Definition
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Stage IV Stage 0–III with platelets <100 × 10 /L Hodgkin’s disease is a primary neoplastic disease of lym-
phoid cells characterised by proliferation of an atypi-
cal form of lymphoid cell termed Reed-Sternberg (RS)
Age
cell.
Peak diagnosis 60–70 years
Incidence
Sex
4per 100,000 per year.
M > F
Age
Pathophysiology
Bimodal distribution with a peak in young adults (15–34
Although there is a proliferation in B cells they have
years) and older individuals (>55).
abnormal function resulting in hypogammaglobuli-
naemia. Staging is shown in Table 12.7.
Sex
2M:1F
Clinical features
Patients may be asymptomatic. Symptoms result from
marrow failure (anaemia, infection and bleeding). On Aetiology
examination there may be lymphadenopathy and hep- Infectious agents particularly Epstein Barr virus have
been implicated. There is an increased incidence in pa-
atosplenomegaly.
tients with HIV. There may be a genetic predisposition.
Investigations
Full blood count reveals a low or normal Pathophysiology

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haemoglobin, a white cell count >15 × 10 /litre of RS cells are a clonal proliferation of B lymphocytes aris-
which at least 40% are lymphocytes, platelets are ing from the germinal centres of lymph nodes. They do
low or normal. Autoimmune haemolytic anaemia/ not produce antibodies. Some of the clinical manifes-
thrombocytopenia are present in 10%. tations are attributed to the production of cytokines.
Blood film demonstrates a high lymphocyte count Oncogeneshavebeenimplicatedinthepathophysiology,

and because these are fragile they appear as smear or upregulation of bcl2 has been shown to be of prognostic
basket cells. significance.
Hypogammaglobulinaemia may be present.

Clinical features
Management Patients present with a painless lymphadenopathy most
CLL frequently requires no treatment other than sup- often within the neck. Fatigue, anorexia, pruritus and al-
portive measures. Symptomatic disease may be treated cohol induced nodal pain may also occur. Involvement
with intermittent chemotherapy such as chlorambucil of mediastinal lymph nodes may cause cough, shortness
or fludarabine. of breath and chest pain. B symptoms may be present
(fever >38 C, drenching night sweats, weight loss of
◦
Prognosis more than 10% within 6 months). On examination there
Prognosis is related to clinical staging. is lymph node enlargement, and hepatic or splenic en-
largement. The staging of Hodgkin’s’s disease is accord-
ing to the Ann Arbor system, which is suffixed by B if
Chronic myelogenous Leukaemia
Bsymptoms are present and A if they are absent (see
See Myeloproliferative disorders page 482. Table 12.8).